GuidelineJournal of the European Academy of Dermatology and Venereology : JEADV2023
S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).
Guideline in Journal of the European Academy of Dermatology and Venereology : JEADV, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 32 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
32 citing papers in PubMed, 1 synthesis or guideline pooled it, 52 citations in OpenAlex.
- European S2k guidelines on management of autoimmune blistering diseases in children and adolescents.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026Guideline
- Lichenoid variant of paraneoplastic pemphigus arising 1 week after thymoma resection.JAAD case reports · 2026Article
- [New treatment options for autoimmune bullous diseases].Dermatologie (Heidelberg, Germany) · 2026Review
- [53/f with painful erosions of the oral mucosa : Preparation for the medical specialist examination: part 62].Dermatologie (Heidelberg, Germany) · 2026Article
- ECMO Support in Paraneoplastic Pemphigus With Respiratory Failure: A Case Report With Immune Cell Monitoring.Clinical case reports · 2026Article
- Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report.Oncology letters · 2026Article
- Paraneoplastic Pemphigus Associated With Renal Cell Carcinoma. Rare Case Report and Literature Review.Clinical medicine insights. Case reports · 2026Article
- Unraveling the autoimmune architecture of pemphigus: from B-cell depletion to network-based immune engineering.Frontiers in immunology · 2026Review
- Case report: Atypical SJS/TEN-like fulminant pemphigus vulgaris: diagnostic challenges and the potential impact of anti-Dsg1 titers and CMV reactivation.Frontiers in immunology · 2026Article
- Clinical improvement of pemphigus-like mucocutaneous disease in a patient with CLL following initiation of zanubrutinib: a case report.Frontiers in immunology · 2026Article
- Paraneoplastic Pemphigus Mimicking Pemphigus Herpetiformis - Overlap of Two Diseases.Acta dermato-venereologica · 2025Article
- Transition from Paraneoplastic Pemphigus to Bullous Pemphigoid after a 26-month Remission.Acta dermato-venereologica · 2025Article
- Paraneoplastic Pemphigus Unveiling Occult Lung Adenocarcinoma: A Rituximab-Responsive Case With Diagnostic and Therapeutic Implications.Clinical case reports · 2025Article
- Review
- A 10-Year Observational Study on Treatment Approaches in Pemphigus and Pemphigoid.Acta stomatologica Croatica · 2025Review
- Autoimmune bullous diseases: pathogenesis and clinical management.Molecular biomedicine · 2025Review
- Paraneoplastic Dermatoses: A Clue for Underlying Malignancies.Journal of clinical medicine · 2025Review
- A case report of Castleman disease with paraneoplastic pemphigus and bronchiolitis obliterans: Challenges and key takeaways.International journal of surgery case reports · 2025Article
- Unicentric Castleman disease with paraneoplastic pemphigus in a young woman: a case report.Frontiers in medicine · 2025Article
- Lung transplantation in a patient with severe bronchiolitis obliterans secondary to Castleman disease.Respiratory medicine case reports · 2025Article
Corrections and comments
- Erratum issued
Authors and funding
54 authors at 20 institutions in 24 countries.
Funding
Abstract
backgroundParaneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans.
objectivesThese guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included.
resultsChronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients.
conclusionsThese are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.